Table 1 Perioperative problems, evaluation items, and correspondences of Rett syndrome patient
From: Anesthetic management of a Rett syndrome patient with apnea and epilepsy: a case report
Perioperative problem of Rett syndrome patients | Preoperative evaluation | Intraoperative management | Postoperative management | |
|---|---|---|---|---|
Central nervous system | Intellectual disability Spasm Autonomic disturbances Variability in sensitivity to anesthetic agents | Mutual understanding may be difficult Control of epilepsy Adjustment of Dosage of antiepileptics | Consider premedication Appropriate analgesia/anesthesia Avoid drugs that induce spasm Prevent hypotension during induction Prevent delayed emergence from anesthesia Appropriate monitoring (BIS/TOF) | Avoid seizure with adequate multimodal analgesia Early resumption/adjustment of anticonvulsants Appropriate monitoring |
Airway | Micrognathia, Trismus Technical difficulties of intubation | Evaluate technical difficulties of intubation | Prepare video laryngoscope or bronchofiber Consider awake intubation | |
Respiratory system | Apnea/abnormality of respiratory pattern Pneumonia Restrictive ventilatory impairment | Preoperative examination Evaluate pneumonia with blood test, X-ray | Avoid apnea with adequate analgesia | Close monitoring of respiratory conditions Physical therapy to avoid respiratory complications NPPV |
Circulatory system | QT prolongation Arrhythmia Bradycardia Structural cardiac defects are rare | Electrocardiogram Echocardiography | Avoid drugs that prolong QT Consider cardiac pacing Monitoring electrocardiogram | Management under ECG monitor |
Nutrition | Malnutrition Hypoalubuminemia Electrolyte abnormalities | Evaluate nutritional status, surgical indication Correct nutritional status, electrolyte abnormalities | Early enteral nutrition | |
Musculoskeletal system | Scoliosis | Appropriate positioning and decompression to prevent bedsores |